Chronic wasting disease of cervids

Curr Top Microbiol Immunol. 2004;284:193-214. doi: 10.1007/978-3-662-08441-0_8.


Chronic wasting disease (CWD) has recently emerged in North America as an important prion disease of captive and free-ranging cervids (species in the deer family). CWD is the only recognized transmissible spongiform encephalopathy (TSE) affecting free-ranging species. Three cervid species, mule deer (Odocoileus hemionus), white-tailed deer (O. virginianus), and Rocky Mountain elk (Cervus elaphus nelsoni), are the only known natural hosts of CWD. Endemic CWD is well established in southern Wyoming and northern Colorado, and has been present in this 'core area' for two decades or more. Apparently CWD has also infected farmed cervids in numerous jurisdictions, and has probably been endemic in North America's farmed deer and elk for well over a decade. Several free-ranging foci distant to the Colorado-Wyoming core area have been discovered since 2000, and new or intensified surveillance may well identify even more foci of infection. Whether all of the identified captive and free-ranging foci are connected via a common original exposure source remains undetermined. Some of this recently observed 'spread' may be attributable to improved detection or natural movements of infected deer and elk, but more distant range extensions are more likely caused by movements of infected captive deer and elk in commerce, or by some yet unidentified exposure risk factor. Research on CWD over the last 5 years has resulted in a more complete understanding of its pathogenesis and epidemiology. CWD is infectious, transmitting horizontally from infected to susceptible cervids. Early accumulation of PrP(CWD) in alimentary tract-associated lymphoid tissues during incubation suggests agent shedding in feces or saliva as plausible transmission routes. Residual infectivity in contaminated environments also appears to be important in sustaining epidemics. Improved tests allow CWD to be reliably diagnosed long before clinical signs appear. Implications of CWD are not entirely clear at this time. Natural transmission to humans or traditional domestic livestock seems relatively unlikely, but the possibility still evokes public concerns; impacts on wildlife resources have not been determined. Consequently, where CWD is not known to occur surveillance programs and regulations that prevent or reduce the likelihood that CWD will be introduced into these jurisdictions should be encouraged. Where CWD is known to occur, affected jurisdictions are conducting surveillance to estimate and monitor trends in geographic distribution and prevalence, managing deer and elk populations in attempts to limit spread, and developing and evaluating techniques for further controlling and perhaps eradicating CWD. Programs for addressing the challenges of CWD management will require interagency cooperation, commitment of funds and personnel, and applied research. Chronic wasting disease (CWD) is perhaps the most enigmatic of the naturally occurring prion diseases. Although recognized as a transmissible spongiform encephalopathy (TSE) since the late 1970s (Williams and Young 1980, 1982), interest in and concern about CWD has only recently emerged. CWD most closely resembles scrapie in sheep in most respects, but recent media and public reaction to CWD has been more reminiscent of that afforded to bovine spongiform encephalopathy (BSE) less than a decade ago. Yet, with the exception of transmissible mink encephalopathy (TME), CWD is the rarest of the known animal TSEs: fewer than 1,000 cases have been diagnosed worldwide, and all but two of these occurred in North America. CWD is unique among the TSEs in that it affects free-living species (Spraker et al. 1997; Miller et al. 2000). The three natural host species for CWD, mule deer (Odocoileus hemionus), white-tailed deer (O. virginianus), and Rocky Mountain elk (Cervus elaphus nelsoni), are all in the family Cervidae and native to North America. Like scrapie, CWD is contagious: epidemics are self-sustaining in both captive and free-ranging cervid populations (Miller et al. 1998, 2000). The geographic extent of endemic CWD in free-ranging wildlife was initially thought to be quite limited and its natural rate of expansion slow; however, recent investigations have revealed that CWD has been inadvertently spread much more widely via market-driven movements of infected, farmed elk and deer. Both the ecological and economic consequences of CWD and its spread remain to be determined; moreover, public health implications remain a question of intense interest. Here, we review current understanding of CWD, its implications, and its management.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Review

MeSH terms

  • Animals
  • Animals, Domestic
  • Animals, Wild
  • Deer*
  • Humans
  • Immunity, Innate / genetics
  • Prion Diseases / epidemiology
  • Prion Diseases / immunology
  • Prion Diseases / transmission
  • Prion Diseases / veterinary*
  • United States / epidemiology
  • Wasting Disease, Chronic / epidemiology*
  • Wasting Disease, Chronic / immunology
  • Wasting Disease, Chronic / transmission